Checklist
for children at risk for progressive hearing lossIt may be very variable with respect to age of onset, rate, severity, frequencies affected, and laterality.
The initially best heard frequencies may deteriorate most.
Some conditions in which progressive hearing loss is a possibility include:
- Intra-uterine infection – especially CMV, Rubella, Syphilis, and HIV
- Genetic- the family history may indicate various degrees of loss
- Chromosomal Syndromes – esp. Downs, Turner, Noonan
- Associated with eye problems, e.g. Usher, Alstrom, Marshall-Stickler syndrome, and mucopolysaccharidosis.
- Associated with renal disease – e.g. Alport, Epstein, tubular acidosis
- Mitochondrial disease – rarely e.g. genetic sensitivity of cochlea to aminoglycosides, Merrf, Melas,
- Ototoxic medication – cisplatin, furosemide, aminoglycosides
- Head injury/trauma/barotrauma
- Noise damage
- Meningitis
- Auto-immune disease e.g. Rheumatoid arthritis
- Middle ear disease – e.g. compounding effects of short-term loss due to glue ear,
- Osteogenesis imperfecta, chondrodysplasias, otosclerosis
Standard referral protocols for Downs, head injury and meningitis exist, but may not always be followed - so be alert!!!